Leber's Idiopathic Stellate Retinopathy

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  • L E B E R ' S I D I O P A T H I C S T E L L A T E R E T I N O P A T H Y


    New Orleans, Louisiana

    Three patients with Leber's idiopathic stellate retinopathya 16-year-old girl, a 26-year-old man, and a 7-year-old girlalso had acute multifocal chorioretinitis. The second patient also had an associated idiopathic peripheral seventh nerve palsy. Extensive neurologic testing and viral cultures gave no explanation of the origin of the retinopathy. However, each of the patients had had an upper respiratory or gastrointestinal illness shortly before the onset of the condition, sug-gesting an infectious origin. In all three patients the optic disk swelling resolved spontaneously and the stellate exudate gradually faded.

    Leber's stellate retinopathy has the fol-lowing features: (1) macular exudates in a star formation; (2) optic disk swelling; (3) unilaterality; (4) spontaneous resolution; and (5) an unknown cause.1 The clinical2 , 3 and fluorescein angiographic features4,5 have been described but, although Gass4 , 6 has suggested a viral cause, the exact nature of this condition remains specula-tive.

    We studied three patients who had the usual clinical features of Leber's stellate retinopathy but who also had multiple foci of chorioretinitis during the active stage of the condition. To the best of our knowledge, this pattern has not been reported previously. One patient also had concurrent unilateral peripheral facial nerve paralysis (Bell's palsy).


    Case 1This 16-year-old girl was first examined in January 1980 because of a 12-day history of decreased

    visual acuity in her left eye. Several days before the onset of reduced vision, she had had a severe system-ic illness resembling influenza and consisting of fever, malaise, and headache. The symptoms had resolved spontaneously, and the patient was well until the visual loss in the left eye began. Medical, ocular, and family histories were noncontributory.

    Our initial examination disclosed an uncorrected visual acuity of R.E.: 6/6 (20/20) and L.E. 6/120 (20/400). There was a relative afferent pupillary de-fect in the left eye. A slit-lamp examination disclosed a clear anterior segment with an occasional cell in the vitreous cavity of the left eye. Ophthalmoscopy of the right eye showed clear media with a normal optic disk and retinal vessels. There were three small focal white lesions in the midperipheral retina. All three lesions were less than 0.25 disk diameter in size and slightly elevated (Fig. 1). They appeared to involve the deeper layers of the retina and the choroid.

    Ophthalmoscopy of the left eye showed 1 + vitre-ous cells. The left optic disk was markedly swollen

    Accepted for publication Oct. 7, 1981. From the Lions Eye Research Laboratories, De-

    partment of Ophthalmology, LSU Eye Center, LSU Medical Center School of Medicine, New Orleans, Louisiana. This study was supported in part by grant EY02377 from the National Eye Institute.

    Reprint requests to Denis M. Carroll, M.D., LSU Eye Center, 136 S. Roman, New Orleans, LA 70112.

    Fig. 1 (Carroll and Franklin). Case 1, right eye. One of three similar chorioretinal lesions (X 2).



    and elevated approximately 2 to 3 mm. The capillar-ies of the optic disk were dilated but no hemorrhages were present. A fine stellate pattern of exudate appeared in the macula with subretinal fluid extend-ing from the optic disk into the macular area. A small, focal, yellow-white, deep retinal lesion, simi-lar to those noted in the right eye, was located midway between the optic disk and the macula (Fig. 2). Visual field examination of the right eye gave normal results, but the left eye demonstrated a dense centrocecal scotoma. A- and B-scan ultrasono-graphic examinations disclosed a solid elevation of the optic nerve head of approximately 2 mm, with a shallow detachment of the retina extending from the optic disk to the macula. The orbits appeared to be normal.

    Fluorescein angiography demonstrated extensive leakage from the left optic disk and late hyperfluores-cence of the focal chorioretinal lesions in both eyes (Fig. 3).

    Tests done when the patient entered the hospital (complete blood cell count, urinalysis, SMA-12, blood cultures, purified protein derivative tuberculin test, histoplasmin skin tests, VDRL test, and chest and skull X-rays with views of the optic foramina) all gave normal results. The patient was discharged after four days and observed but not treated.

    A follow-up examination two weeks later disclosed a visual acuity of R.E.: 676 (20/20) and L.E.: 6/120 (20/400). The focal chorioretinal lesions in the right eye remained unchanged. The optic nerve swelling had lessened, and there was a marked reduction in the amount of subretinal fluid in the left eye. The macular star figure was still present.

    Examination four weeks after onset disclosed that the visual acuity in the left eye had improved to 6/60 (20/200). The focal chorioretinal lesions in the right eye had faded slightly but were still visible. Further reduction in the optic nerve swelling in the left eye had occurred, along with resolution of all subretinal fluid. The macular star figure and focal chorioretinal lesion remained. Visual field examination showed that the centrocecal scotoma was reduced in size.

    The patient was examined two months later in April 1980; at that time she reported that her vision had improved steadily. Her visual acuity had im-proved to R.E.: 674.5 (20/15) and L.E.: 6/60 +1 (20/200 +1). The afferent pupillary defect was still present in the left eye. Ophthalmoscopy of the right eye showed only faint white spots that corresponded to the previous focal chorioretinal lesions. Examina-tion of the left eye disclosed a flat optic disk nasally, with pale swelling of the temporal edge and slight swelling of the adjacent nerve fiber layer. The sub-retinal fluid was entirely absent, and only small punctate areas of intraretinal exudate remained in the macula.

    Case 2This 26-year-old man was first examined in August 1980 because of a 12-day history of blurred vision in the left eye and a three-day history of left-sided facial weakness. He had noted stomach cramps, diarrhea, and malaise just before the onset of visual loss. Medical, ocular, and family histories were noncontributory. Our initial examination disclosed an uncorrected visual acuity of R.E.: 6/4.5 1 (20/15 - 1 ) and L.E.: 6/24 - 1 (20/80 - 1 ) . Refraction in the left eye improved the visual acuity to 6/15 12 (20/50) with a 1.00 sphere. Ocular motility was

    Fig. 2 (Carroll and Franklin). Case 1, left eye. Note the markedly swollen optic disk, the macular star figure, and the focal white chorioretinal lesion be-tween the optic disk and the macula.

    Fig. 3 (Carroll and Franklin). Case 1, right eye. Late-phase fluorescein angiogram shows hyperfluo-rescence of two focal chorioretinal lesions. The upper lesion is the one shown in Figure 1.


    Fig. 4 (Carroll and Franklin). Case 2, left eye. Note the mild optic disk swelling and the macular star figure.

    Fig. 5 (Carroll and Franklin). Case 2, left eye. There are two focal chorioretinal lesions. The periph-eral lesion appears to be more atrophic.

    normal, but there was a complete left-sided peripher-al facial nerve palsy. Intraocular pressure by applana-tion was R.E.: 17 mm Hg and L.E.: 12 mm Hg. The pupils appeared to be normal and no definite afferent pupillary defect was present. A slit-lamp examination disclosed a clear anterior segment in both eyes. Ophthalmoscopy showed clear media with a normal optic disk, macula, and retina in the right eye and clear media in the left eye. The left optic disk was swollen on the temporal side, with a small flame-shaped hemorrhage at the 5 o'clock position. There was a fine macular star figure but no definite subreti-nal fluid in the macular area (Fig. 4). Two focal yellow-white lesions less than 0.25 disk diameter in size were present inferotemporal to the macula (Fig. 5). The peripheral lesion was yellow with a diffuse border and involved the deep and superficial layers of the retina. The central lesion was white with sharper borders and involved only the deeper retina. There were no cells in the vitreous cavity.

    Fluorescein angiography demonstrated diffuse leakage from the left optic disk and a normal macula. The peripheral focal chorioretinal lesion showed hy-perfluorescence early with no dye leakage and was consistent with a retinal pigment epithelial defect. The proximal chorioretinal lesion became progres-sively hyperfluorescent in the later stages of the angiogram (Fig. 6).

    When the patient was admitted to the hospital, a complete neurologic examination disclosed a periph-eral seventh nerve weakness. Results of a general physical examination were normal. The white blood cell count was 6,800 cells/mm3 with 1% basophils, 6% eosinophils, 3% monocytes, 24% lymphocytes, 26% polymorphonuclear leukocytes, and normal platelets. The red blood cell count, hemoglobin level, and

    hematocrit were normal. The antinuclear antibody was positive (1:20). Serum electrolyte, blood urea nitrogen, glucose, liver function, urinalysis, thyroid (T3 and T4), and VDRL tests were normal, as were skull X-ray films. Plain films of the sinuses disclosed evidence of sinusitis involving both the maxillary and the right ethmoid sinuses. Computed axial tomogra-phy of the brain and orbits demonstrated evidence

    Fig. 6 (Carroll and Franklin). Case 2, left eye. Late-phase fluorescein angiogram of the two focal chorioretinal lesions shown in Figure 5.

  • VOL. 93,